Monday, December 17, 2018

Week 278: Poop in a hat for science

I knew this wasn't going to be our greatest clinic visit ever, but even so, getting our numbers was a bit disheartening. We went into September with Lemon at the 95th percentile for BMI. As of last week, he'd lost a total of 6 pounds, and also grown taller. We have done this math in previous winters but I don't think the result has ever been so striking. He has fallen all the way down to the 43rd percentile for BMI (when the goal is always to stay above 50th). A lot of hard work, all torched in 3 months.

The "good" news, though, is as follows. First, his height velocity is undiminished, so even though he's lost a lot of weight, he has still had enough nutrition/reserves to keep growing upward. Second, his lung function (however reliable pulmonary function tests are in kindergarteners) is unchanged and at 100% of expected for his size. Third, we know what we need to do and we have the tools to do it (more calories in, through the tube). Fourth, as our nurse-practitioner pointed out, the fact that we had those reserves on board probably saved us from a hospital admission and IV antibiotics. So, it's not all grim news.

We're following up with another visit in 2 months to see if going back up to 3.5 cartons of formula per night from the 2.5 we had switched to is enough to get things moving in the right direction again. I suspect it will be, as long as we can actually do it every night. Luckily, winter vacation starts on Friday so that should really cut down on the number of viruses that Lemon is exposed to, and also should increase the opportunities he has to eat. I can't say that I'm shocked that he doesn't eat much lunch, given that the school lunch period is 15 minutes. But, if he's at home and can graze on home-made holiday treats all day long for two weeks, we may get somewhere.

We also found out that it was time for everyone's favorite part of the clinical study that Lemon is participating in: stool sample collection. Even better--they wanted another sample from the non-CF sibling. This is the first time that I've had to collect samples from two toilet trained individuals, who, I might add, are at peak scatological humor. It make take our household several weeks to recover from the hilarity of scientists asking me to place a plastic "hat" in our toilet to catch the precious specimens, followed by the process of scooping the right amount of the goods into a little vial and putting the vial in a bag, the bag in the container, the containers in the mailing envelope and, of course, the biggest question, "Will the mailman know it's poop?"

That excitement behind us, I also signed Lemon up to participate in two more tests for the study, a multiple breath washout test and a chest CT. I'm always glad to participate in whatever research we can, because this is the way forward for the whole CF community. For these tests, though, I'm also really curious about the results. These are tests we wouldn't normally have access to as part of routine care, but I hope they will give us a more complete picture than we've had before of what's going on in Lemon's lungs. That is, if I can convince him to lie still for 20 minutes in a CT scanner. Wish me luck!

Monday, December 10, 2018

Week 277: Perchance to dream

The nights are long at this time of year, so you would think everyone would be well rested. I think every parent of a kid with CF will tell you that a good night's rest is hard to come by. Lemon had to give up his nap when he started kindergarten. Yes, he was still napping at age 5. My totally non-random sample of other CF parents is pretty consistent--kids with CF need a lot of sleep. Anyhow, since he gave up his nap, we now have to wake him up every morning so we can do everything we need to do in time to start our day, which kills me. And that's after 11 hours of sleep. Today, there was no school, so Lemon was home all afternoon with his brother and the babysitter. And of any possible activity he could have chosen, what did he pick? A 2-hour nap. As in he voluntarily walked to his room, lay down, and didn't come out for 2 hours.

Here is another unexpected source of sleep disruption: the kids decided this weekend that they wanted to rearrange their bedrooms. Both of their beds had been against one wall of their rooms, and we rotated them 90 degrees so they were against the other walls. Presto, a whole fresh look on life. One small issue, which we hadn't thought about when we made this change: Lemon's pole and formula pump were to his left in the old configuration, and are on his right in the new configuration. So, last night, his formula pump kept alarming because he was rolling over on top of the tubing and cutting of the flow, something that almost never happened in our old configuration. Luckily, the only person who is woken up by the alarm is me (loose definition of "luckily"). We're trying again tonight, so fingers crossed that even though the pump doesn't wake him up, maybe it reprograms his subconscious somehow so that he'll start rolling in the other direction.

Unrelated to sleep, but there's another interesting thing that happened recently. We have a copay card for one of Lemon's medicines. Yay! We've had the same card, with the same numbers on it, for several years. Awesome! The pharmacy keeps those numbers on file so that the discount is applied every time that we fill that prescription. What could be better? Well, the company that makes the medicine sent us a letter, on a single 8.5*11 piece of regular paper, saying that, great news, they're making "improvements" to the copay plan to serve us better (ominous). No significant hints at why the new plan will be better, or anything like that, just that it is now better.

At the bottom, in an area about the size of the surface of a matchbook, are some numbers that look like the numbers on our card. I think nothing of it, because I assume they're just reprinting the numbers on our card so that we know the letter was intended for us or something. Luckily, Papa Bear has a more suspicious mind than I do, so he got out our old card and discovered that the only discernible "improvement" in the copay plan is that we now have some totally different numbers than the ones we used to have, even though there was nothing in the letter to the effect of "Hey, guess what, here are your new and improved numbers that you will need to use to get your new and improved benefits!" Being the crafty sort, Papa Bear excised this matchbook-sized area from the letter and affixed it to our old copay card with tape so he will have it when inevitably we are denied our copay benefit because our numbers no longer match what the drug company will have on file. New and improved, I tell you.

Monday, December 3, 2018

Week 276: For the duration

With Papa Bear safely back home from his recent trip to Copenhagen (yeah, don't cry too many tears on his behalf), we are FINALLY done with the 2018 travel season. We are all staying right here until sometime next year. It has been a rather dizzying fall but, somehow, we all made it through.

In health news, both kids seem to have contracted yet another cold. How many different respiratory viruses are there in Wisconsin? The answer appears to fall somewhere between "several" and "one trillion." Lemon did have a self-advocacy breakthrough on Friday, though. After a day of coughing (a lot, but by far not the worst we've ever had), I set up his formula as usual. He eyed the bag nervously as I hung it, and said "Mom, that looks like too much formula. I might throw up." So we skipped it. And do you know who did not puke on Friday night? Lemon. And guess who didn't have to wash any puke out of any sheets on Saturday? Me. Win all around. Except for weight gain, of course, which remains non-existent. But, hey, it's hard to win on all fronts at the same time, especially in the winter. I'll take a partial victory.

In other news, Vertex released some great data on a Phase III trial of their triple combination therapy (the one that would help Lemon). You can read more here. They saw big improvements in lung function of people with one or two copies of the common F508del mutation. They are still waiting for the results of a Phase III trial with their second triple combination before deciding which (or both) drug combinations to submit to the FDA, probably late next year. And then, hopefully, they will begin enrolling a pediatric trial, and hopefully we will be in it!

Speaking of being in it, I forgot to mention a couple of weeks ago something interesting that came up during our appointment with GI. The GI doc asked me if Lemon was eligible for any of the current Vertex drugs, and I said no, but that I was hoping to get him enrolled in a future trial of one of the triple combinations. And he said, "Oh, good thing we got his LFTs back in the normal range, then, and we'll have to keep an eye on that so he stays eligible." And you know, I've read the enrollment criteria for the trials many times, and I know that they require the liver values to be at or near normal, and somehow I had never connected the dots in my head that Lemon would not have been eligible before we got him on ursodiol, because his liver values would have been considered too high. Huh. Interesting that our CF clinic wasn't thinking about that. Anyhow, here's to having a variety of different specialists on the care team.

Somehow I've spent an inordinate amount of time this week planning for both Christmas and next summer. Coordinating the kids schedules, figuring out how Lemon can do a little 3-day art program over the holidays without my having to train anyone new on doing enzymes, trying to decide who will be doing what in the summer when almost any camp will take someone who is 5 and almost no camps will take someone who is 3...the party never stops!

Monday, November 26, 2018

Week 275: Road tripping

Not to get too self-congratulatory, but I believe this may be our family's first trip where we didn't forget something important. I always feel like we couldn't have forgotten anything important, because it always looks like we're packed for intergalactic travel when we leave town even for a night. Inevitably, though, something small gets left behind. This time, though, I actually got everything. You might reasonably suggest that I should develop a packing list of some kind that lists all the CF-related stuff that we need to bring on every trip. You would be correct that this would be a good idea. And I fully intend to do it, right after I set up my calendar reminders about changing out the filter on the compressor for the vest.

Speaking of changing out that filter, holy smokes. Um, yeah, I guess it really did need to be changed (top = new, bottom = old). Don't worry, though. All that sketchy-looking white stuff that's on the filter? It's almost all pure salt from the 7% saline we nebulize twice a day. Honestly I'm amazed the thing still runs given how much salt must be inside it.

Anyhow, in addition to having everything we needed with us, Thanksgiving was downright awesome. We went to Minneapolis and stayed at a nice Airbnb. We spent the bulk of the weekend with some of my oldest and dearest friends, who now live out in CA but were in Minnesota to see their relatives. It reminded me so much of the Thanksgivings of my childhood--a pack of children running wild, tons of food, laughter, conversation, tryptophan comas. Just fantastic, by far the best Thanksgiving we've had since we moved to Wisconsin.

We also took advantage of our time in Minneapolis to do other fun things, such as visit the science museum, the aquarium, and one of Lemon's oldest friends (saying this cracks me up). His little buddy moved to Minneapolis over the summer, but they picked up right where they left off at the end of 4K, disappearing into the attic playroom for several hours. Lemon was very gleeful about the fact that we didn't know what they were doing while they were up there. Evidence strongly suggests that they were decorating old shoe boxes with glitter glue and markers, but I couldn't say for certain.

The only little dark cloud in the sky at the moment is weight gain, or rather the lack thereof. Despite having been healthy for a month, Lemon has not gained back even an ounce of the weight that he lost in September and October. I went back up to 3 cartons of formula a night, along with my trusty DuoCal, to see if that would help. No luck so far, and now I'm burning through my stash of formula because our order was changed to 2.5 cartons per day, so to do 3 I have to use up what I had saved from the times he was sick. We have a clinic visit coming up in a couple of weeks so if we haven't gotten anywhere by then I think I will have to ask the dietitian to change our order again. Lemon is still in around the 60th percentile for BMI, so definitely not in any serious trouble, but he does keep getting taller. And when you get taller without gaining any weight, well. We've played this game before. I think the great lesson from this fall is that fall is not a good time to back off on tube feeds. Previous seasons that we have lived through seem to suggest that spring, summer, and winter are also not good times to back off on tube feeds. I'm beginning to detect a pattern, I think...

Monday, November 19, 2018

Week 274: Cadence

Treating CF means keeping up with a lot of things. I would give us a gold star for keeping up with the things that happen on a daily basis. We do all the treatments with the vest. All the inhalers. All the nebulized stuff. All the pills. All the enzymes. Every day. I would even go so far as to say that we (well, Papa Bear specifically) are pretty good at the stuff that needs to happen every month, like the calls with the pharmacy to get refills on all the meds. We even hit the quarterly stuff pretty solidly, which is mainly our clinic visits.

But, I think if the interval gets longer than quarterly, things start to get a little raggedy. For example, changing the g-tube button. I think that's supposed to be every 3-6 months. So, I guess maybe I should do it quarterly and then I would be good at it. Instead, I usually just wait until the button is so old that it can't be connected to an extension anymore, or until Lemon pops it out while doing acrobatics, and I take that as a sign to replace it. And the nebulizer cups and masks. Those are supposed to be replaced every 6 months. I always think _this_ will be the time that I note something on some kind of electronic calendar so that it will remind me in 6 months that it's time for a fresh set. Then, inevitably, I forget to set the reminder, so I just keep going with the set we have until the masks literally crack apart from being sterilized too many times. I tell myself that this cracking always happens right around the 6-month mark, so it's a good indicator, but actually I have no idea. It could be 6  months, but could just as well be 4 or 10, I really couldn't say.

Then there's the stuff that happens even more infrequently, like annually. For example, our annual visit to the GI doctor. Of course, doctor's calendars are only open something like 6 months out, so you can't do anything convenient like schedule your one-year follow up as you're leaving your current appointment. You are told to call back in 6 months. Hah. I did eventually remember to call, but by the time I called the doctor's calendar was filled up for 3 months, so we didn't actually get in to see him until this past Friday. As I had promised Lemon, it was a total nothing appointment anyhow. The doctor asked us how things were going, I told him, he pressed on Lemon's belly a bit, and we were off again. He does want us to repeat the liver function tests in December so we can see how Lemon is doing on the new dose of ursodiol, and then we can follow up in (you guessed it) a year.

The other thing that happens maybe even less frequently than annually is replacing Lemon's vest. He's had his current one for a long time. How long? Who knows. I had been looking at it every now and again and thinking he'd gained so much height that he probably needed another one. And then that thought would leave my mind as soon as I'd left his room. But, the vest company had a rep at the CF education day that I went to last weekend, and she gave me a flier with the 800 number on it and pictures of all the different patterns of fabric that they have. So, I finally managed to sit down with Lemon, get him to pick a fabric, and placed the order, which arrived today. Lemon was thrilled, and I could tell watching him do his therapy today that the new vest really fits him much better than the old. So, I'm glad I finally got myself together to do it. Also included in the box with the vest was a new filter for the compressor that goes with the vest. Here's some new information I just learned. That filter is supposed to be changed...quarterly.

Monday, November 12, 2018

Week 273: Meet a hero

You know what's great after a lot of traveling? Not traveling. Doing laundry and putting your clean clothes in the dresser rather than back in the suitcase. Being able to honestly respond "Nope!" when your 3-year-old asks, "Mama, are you going on another trip?" 

One really nice thing that I was able to do here at home was to attend our annual CF Family Education Day on Saturday. I always love the chance to catch up with the other families, the people who know exactly what my life is really like and that I never see, even though we live in the same state. It's great to hear in person how everyone's kids are doing, and swap tips, advice, and tears. As an added bonus, we got to taste free samples of the new s'mores flavor of Pediasure. Verdict: pure nast, kind of like a liquid PowerBar. But, some kids love it, apparently.

Another great feature of the day was the opportunity to meet an adult with CF. This year's speaker was a man from WI who is in his mid 30's, married, works as a phys ed teacher and basketball coach, and is 6'1", 195lb (!!!). He had an incredibly positive outlook on life and it was so wonderful to see a real-life example of what could be possible for Lemon. 

Perhaps the highlight this year was the chance to meet Robert Beall, the former president and CEO of the CF Foundation. He was with the foundation for an incredible 35 years, retiring in 2015. One of the things he brought with him to set the stage was a sort of medical manual about CF that was published in 1965. Suffice it to say, the picture then was unbelievably grim, and the photos of the children in the book were absolutely heartbreaking. 

But, there were a group of desperate and committed parents who wanted to push things forward. They started incredibly small, with a single research grant of $10,000. When Dr. Beall joined the foundation, things were incrementally less grim than in 1965, but not much. Most kids with CF didn't make it past 10 years of age. The amount of change that has happened since that time (in no small part thanks to Beall's leadership) is staggering. To see Dr. Beall, who is now an older guy (and not very tall) be absolutely dwarfed by an adult man with CF, when there were no such people alive at the time Dr. Beall joined CFF, is so moving and gives me so much hope for Lemon's future. I feel very fortunate to have had the chance to see Dr. Beall in person and to shake his hand, given that he is now retired and doesn't travel much anymore. A true once-in-a-lifetime opportunity, and I was glad to be in town to take advantage of it.  

Monday, November 5, 2018

Week 272: Back to where you once belonged

I am back. In perhaps the most insane travel period of my post-kids life, I spent the night in each of the 4 time zones in the contiguous 48 states within a one-week period. San Diego, Denver, Madison, Indianapolis. I am done. No more traveling for a while. Papa Bear has been an absolute rock during all this insanity, but his reserves are pretty much tapped out, and the kids are definitely feeling like they need there mom around more. And I for one am done with being on the road and in hotels and all that. Not that it hasn't all been fun, spending a bunch of face-to-face time with my coworkers and having an absolute blast at the Indianapolis Monumental Marathon with my ace marathoning buddy, but man I am ready to just be HOME. Also I ran an all-time best of 3:43 an my legs are tired.

In spite of all of my gallivanting around, things seem to have held together on the home front reasonably well. All of the boys seem to have had a cold of varying degrees of severity, but Lime is almost completely well again, Lemon is very congested but otherwise in fine spirits, and Papa Bear is thoroughly exhausted but may yet pull through. The cats are still present, if slightly starved for attention.

One interesting development that appears to have taken place is the onset of some nutrition lessons at Lemon's school. When I got back from Indianapolis on Sunday, I took the kids grocery shopping, and asked Lemon if there was anything he wanted me to get for him for his lunches. He informed me that he wasn't going to drink juice boxes anymore, because juice was bad for his teeth. He also said that he was done with chips, since they are not healthy. But, juice and chips are the only things he reliably consumes during the day, and we need every calorie! I totally appreciate the overall intent, but I feel like some recognition of the fact that everyone has different dietary needs might also be in order. After all, in spite of the juice and the chips and the tube feeds, we are still pounds away from where we were before school started, and we're desperate to regain every ounce. Fortunately school conferences are coming up in a couple of weeks so I'll have a good opportunity to discuss with Lemon's teacher.

Speaking of discussions with Lemon's teacher, I have noticed that it is now November, and back in July we thought we were going to have a meeting about Lemon's 504 plan in October. I guess that ship has sailed, but I suppose now that I am actually here for a couple of months I should try to get something on the calendar before the end of the year.

Finally, I know you all have had it coming at you from every possible direction already, but the midterm elections are tomorrow. VOTE.